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pseudohermaphroditism |
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pseudohermaphroditism /pseu·do·her·maph·ro·dit·ism/ (-her-maf´ro-dīt-izm″) a state in which the gonads are of one sex, but one or more contradictions exist in the morphologic criteria of sex. In female p., the individual is genetically female and has female gonads (ovaries) but has significant male secondary sex characters. In male p., the individual is genetically male and has male gonads (testes) but has significant female secondary sex characters.
pseudohermaphroditism [-hərmaf′rəditiz′əm] Etymology: Gk, pseudes + Hermaphroditos, son of Hermes and Aphrodite a condition in which a person exhibits the somatic characteristics of both sexes though possessing the physical characteristics of either males (testes) or females (ovaries). Also spelled pseudohermaphrodism. See also ambiguous genitalia, feminization, def. 2, hermaphroditism. pseudohermaphroditic, adj. pseudohermaphroditism a state in which the gonads are of one sex but one or more contradictions exist in the morphological criteria of sex. In female pseudohermaphroditism, the individual is a genetic and gonadal female with partial masculinization; in male pseudohermaphroditism, the individual is a genetic and gonadal male with incomplete masculinization. Pseudohermaphroditism is not to be confused with hermaphroditism, in which the individual possesses both ovarian and testicular tissue. pseudohermaphroditism Endocrinology A state in which a person has the gonadal tissue of one sex, but the wiring, plumbing, and/or chassis of the opposite sex. See Female pseudohermaphroditism, Male pseudohermaphroditism. Cf Hermaphroditism, Intersex, Virilization.
Pseudohermaphroditism
Female pseudohermaphroditism A condition affecting a genotypic–46, XX female with ovaries, caused by a relative excess of androgen in utero, resulting in equivocal or masculinized genital duct derivatives, ie external genitalia and/or a
male phenotype with genital ambiguity and/or virilization Etiology 1. Adrenogenital syndrome Defects of 21-hydroxylase, 11-β-hydroxylase or 3-β-hydroxysteroid dehydrogenase, or delta 5-4 isomerase deficiency, resulting in ↑ androgenic
intermediates 2. Maternal ingestion of progestins or androgens and three maternal virilizing tumors–eg, luteoma of pregnancy
Male pseudohermaphroditism, A condition affecting a genotypic–46, XY male with testes, caused by a relative deficiency of androgen in utero, resulting in a phenotypic female with ambiguous genitalia Etiology 1. Gonadal defects
Testicular regression syndrome, persistent müllerian duct origin, Leydig cell agenesis and defects in testosterone synthesis 2. End-organ defects Testicular feminization or androgen insensitivity syndrome, incomplete androgen insensitivity
syndrome and 5-α reductase deficiency. See Testicular feminization
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